Vesicles and bullae frequently accompany, or precede by a short period, circumscribed lesions of mastocytosis occurring in infancy or early childhood. Bullae, in association with diffuse cutaneous involvement and a varying functional disturbance, in rare examples, continue for many months or even years. Significant systemic involvement, and the ultimate prognosis, may relate to the age of onset of the condition. Comparison is made to other chronic bullous disorders of infancy and early childhood.

This review of bullous mastocytosis is based upon intensive study of a child, who demonstrated cyclic patterned exacerbations of a bullous diffuse dermatosis at times accompanied by a febrile seizure-like state. Cytologic, histopathologic (skin, lymph node, liver, and duodenum), histochemical biochemical, metabolic, and electron microscopic techniques were utilized. Opinions are expressed on the physiologic and biologic significance of the observations.